Gene CFTR : cystic fibrosis transmembrane conductance regulator (ATP-binding cassette sub-family C, member 7) in Sus scrofa

See the equivalent entry at NCBI

In other species: taurine cattle , sheep , white-tufted-ear marmoset , domestic ferret , rabbit

Symbol: CFTR

Synonyms: ABCC7

Description: cystic fibrosis transmembrane conductance regulator (ATP-binding cassette sub-family C, member 7)

Type of gene: protein-coding

NCBI gene id: 403154

Other designations: cystic fibrosis transmembrane conductance regulator|ATP-binding cassette sub-family C member 7|ATP-binding cassette transporter sub-family C member 7|cAMP-dependent chloride channel|channel conductance-controlling ATPase

Links: Homologene , Ensembl

Genomic location: 18:28818209..28627717 [Chromosome accession NC_010460.4]

Related phenes:

OMIA:001794-9823 : Cystic fibrosis in Sus scrofa


Note: the references are listed in reverse chronological order (from the most recent year to the earliest year), and alphabetically by first author within a year.
2013 Potash, A.E., Wallen, T.J., Karp, P.H., Ernst, S., Moninger, T.O., Gansemer, N.D., Stoltz, D.A., Zabner, J., Chang, E.H. :
Adenoviral gene transfer corrects the ion transport defect in the sinus epithelia of a porcine CF model. Mol Ther 21:947-53, 2013. Pubmed reference: 23511247. DOI: 10.1038/mt.2013.49.
Reznikov, L.R., Dong, Q., Chen, J.H., Moninger, T.O., Park, J.M., Zhang, Y., Du, J., Hildebrand, M.S., Smith, R.J., Randak, C.O., Stoltz, D.A., Welsh, M.J. :
CFTR-deficient pigs display peripheral nervous system defects at birth. Proc Natl Acad Sci U S A 110:3083-8, 2013. Pubmed reference: 23382208. DOI: 10.1073/pnas.1222729110.
2012 Chang, E.H., Pezzulo, A.A., Meyerholz, D.K., Potash, A.E., Wallen, T.J., Reznikov, L.R., Sieren, J.C., Karp, P.H., Ernst, S., Moninger, T.O., Gansemer, N.D., McCray, P.B., Stoltz, D.A., Welsh, M.J., Zabner, J. :
Sinus hypoplasia precedes sinus infection in a porcine model of cystic fibrosis. Laryngoscope 122:1898-905, 2012. Pubmed reference: 22711071. DOI: 10.1002/lary.23392.
Collawn, J.F., Matalon, S. :
The role of CFTR in transepithelial liquid transport in pig alveolar epithelia. Am J Physiol Lung Cell Mol Physiol 303:L489-91, 2012. Pubmed reference: 22797251. DOI: 10.1152/ajplung.00216.2012.
Klymiuk, N., Mundhenk, L., Kraehe, K., Wuensch, A., Plog, S., Emrich, D., Langenmayer, M.C., Stehr, M., Holzinger, A., Kröner, C., Richter, A., Kessler, B., Kurome, M., Eddicks, M., Nagashima, H., Heinritzi, K., Gruber, A.D., Wolf, E. :
Sequential targeting of CFTR by BAC vectors generates a novel pig model of cystic fibrosis. J Mol Med (Berl) 90:597-608, 2012. Pubmed reference: 22170306. DOI: 10.1007/s00109-011-0839-y.
Pezzulo, A.A., Tang, X.X., Hoegger, M.J., Alaiwa, M.H., Ramachandran, S., Moninger, T.O., Karp, P.H., Wohlford-Lenane, C.L., Haagsman, H.P., van Eijk, M., Bánfi, B., Horswill, A.R., Stoltz, D.A., McCray, P.B., Welsh, M.J., Zabner, J. :
Reduced airway surface pH impairs bacterial killing in the porcine cystic fibrosis lung. Nature 487:109-13, 2012. Pubmed reference: 22763554. DOI: 10.1038/nature11130.
Potton, E. :
Reduced pH in the airways alters bacterial killing in CFTR-/- porcine lungs. Thorax , 2012. Pubmed reference: 23125169. DOI: 10.1136/thoraxjnl-2012-202842.
Uc, A., Giriyappa, R., Meyerholz, D.K., Griffin, M., Ostedgaard, L.S., Tang, X.X., Abu-El-Haija, M., Stoltz, D.A., Ludwig, P., Pezzulo, A., Abu-El-Haija, M., Taft, P., Welsh, M.J. :
Pancreatic and biliary secretion are both altered in cystic fibrosis pigs. Am J Physiol Gastrointest Liver Physiol 303:G961-8, 2012. Pubmed reference: 22936270. DOI: 10.1152/ajpgi.00030.2012.
2011 Abu-El-Haija, M., Sinkora, M., Meyerholz, D.K., Welsh, M.J., McCray, P.B., Butler, J., Uc, A. :
An activated immune and inflammatory response targets the pancreas of newborn pigs with cystic fibrosis. Pancreatology 11:506-15, 2011. Pubmed reference: 22057257. DOI: 10.1159/000332582.
Chang, E.H., Lacruz, R.S., Bromage, T.G., Bringas, P., Welsh, M.J., Zabner, J., Paine, M.L. :
Enamel pathology resulting from loss of function in the cystic fibrosis transmembrane conductance regulator in a porcine animal model. Cells Tissues Organs 194:249-54, 2011. Pubmed reference: 21525720. DOI: 10.1159/000324248.
Cho, H.J., Joo, N.S., Wine, J.J. :
Defective fluid secretion from submucosal glands of nasal turbinates from CFTR-/- and CFTR (ΔF508/ΔF508) pigs. PLoS One 6:e24424, 2011. Pubmed reference: 21935358. DOI: 10.1371/journal.pone.0024424.
Fisher, J.T., Zhang, Y., Engelhardt, J.F. :
Comparative biology of cystic fibrosis animal models. Methods Mol Biol 742:311-34, 2011. Pubmed reference: 21547741. DOI: 10.1007/978-1-61779-120-8_19.
Keiser, N.W., Engelhardt, J.F. :
New animal models of cystic fibrosis: what are they teaching us? Curr Opin Pulm Med 17:478-83, 2011. Pubmed reference: 21857224. DOI: 10.1097/MCP.0b013e32834b14c9.
Ostedgaard, L.S., Meyerholz, D.K., Chen, J.H., Pezzulo, A.A., Karp, P.H., Rokhlina, T., Ernst, S.E., Hanfland, R.A., Reznikov, L.R., Ludwig, P.S., Rogan, M.P., Davis, G.J., Dohrn, C.L., Wohlford-Lenane, C., Taft, P.J., Rector, M.V., Hornick, E., Nassar, B.S., Samuel, M., Zhang, Y., Richter, S.S., Uc, A., Shilyansky, J., Prather, R.S., McCray, P.B., Zabner, J., Welsh, M.J., Stoltz, D.A. :
The ΔF508 mutation causes CFTR misprocessing and cystic fibrosis-like disease in pigs. Sci Transl Med 3:74ra24, 2011. Pubmed reference: 21411740. DOI: 10.1126/scitranslmed.3001868.
Pierucci-Alves, F., Akoyev, V., Stewart, J.C., Wang, L.H., Janardhan, K.S., Schultz, B.D. :
Swine models of cystic fibrosis reveal male reproductive tract phenotype at birth. Biol Reprod 85:442-51, 2011. Pubmed reference: 21593481. DOI: 10.1095/biolreprod.111.090860.
2010 Joo, N.S., Cho, H.J., Khansaheb, M., Wine, J.J. :
Hyposecretion of fluid from tracheal submucosal glands of CFTR-deficient pigs. J Clin Invest 120:3161-6, 2010. Pubmed reference: 20739758. DOI: 10.1172/JCI43466.
Li, H., Ganta, S., Fong, P. :
Altered ion transport by thyroid epithelia from CFTR(-/-) pigs suggests mechanisms for hypothyroidism in cystic fibrosis. Exp Physiol 95:1132-44, 2010. Pubmed reference: 20729267. DOI: 10.1113/expphysiol.2010.054700.
Meyerholz, D.K., Stoltz, D.A., Pezzulo, A.A., Welsh, M.J. :
Pathology of gastrointestinal organs in a porcine model of cystic fibrosis. Am J Pathol 176:1377-89, 2010. Pubmed reference: 20110417. DOI: 10.2353/ajpath.2010.090849.
Plog, S., Mundhenk, L., Bothe, M.K., Klymiuk, N., Gruber, A.D. :
Tissue and cellular expression patterns of porcine CFTR: similarities to and differences from human CFTR. J Histochem Cytochem 58:785-97, 2010. Pubmed reference: 20498480. DOI: 10.1369/jhc.2010.955377.
Stoltz, D.A., Meyerholz, D.K., Pezzulo, A.A., Ramachandran, S., Rogan, M.P., Davis, G.J., Hanfland, R.A., Wohlford-Lenane, C., Dohrn, C.L., Bartlett, J.A., Nelson, G.A., Chang, E.H., Taft, P.J., Ludwig, P.S., Estin, M., Hornick, E.E., Launspach, J.L., Samuel, M., Rokhlina, T., Karp, P.H., Ostedgaard, L.S., Uc, A., Starner, T.D., Horswill, A.R., Brogden, K.A., Prather, R.S., Richter, S.S., Shilyansky, J., McCray, P.B., Zabner, J., Welsh, M.J. :
Cystic fibrosis pigs develop lung disease and exhibit defective bacterial eradication at birth. Sci Transl Med 2:29ra31, 2010. Pubmed reference: 20427821. DOI: 10.1126/scitranslmed.3000928.
Widdicombe, J.H. :
Transgenic animals may help resolve a sticky situation in cystic fibrosis. J Clin Invest 120:3093-6, 2010. Pubmed reference: 20739746. DOI: 10.1172/JCI44235.
2009 Welsh, M.J., Rogers, C.S., Stoltz, D.A., Meyerholz, D.K., Prather, R.S. :
Development of a porcine model of cystic fibrosis. Trans Am Clin Climatol Assoc 120:149-62, 2009. Pubmed reference: 19768173.
2008 Liu, Y., Wang, Y., Jiang, Y., Zhu, N., Liang, H., Xu, L., Feng, X., Yang, H., Ma, T. :
Mild processing defect of porcine DeltaF508-CFTR suggests that DeltaF508 pigs may not develop cystic fibrosis disease. Biochem Biophys Res Commun 373:113-8, 2008. Pubmed reference: 18555011. DOI: 10.1016/j.bbrc.2008.06.009.
Rogers, C.S., Hao, Y., Rokhlina, T., Samuel, M., Stoltz, D.A., Li, Y., Petroff, E., Vermeer, D.W., Kabel, A.C., Yan, Z., Spate, L., Wax, D., Murphy, C.N., Rieke, A., Whitworth, K., Linville, M.L., Korte, S.W., Engelhardt, J.F., Welsh, M.J., Prather, R.S. :
Production of CFTR-null and CFTR-DeltaF508 heterozygous pigs by adeno-associated virus-mediated gene targeting and somatic cell nuclear transfer. J Clin Invest 118:1571-7, 2008. Pubmed reference: 18324337. DOI: 10.1172/JCI34773.
Rogers, C.S., Stoltz, D.A., Meyerholz, D.K., Ostedgaard, L.S., Rokhlina, T., Taft, P.J., Rogan, M.P., Pezzulo, A.A., Karp, P.H., Itani, O.A., Kabel, A.C., Wohlford-Lenane, C.L., Davis, G.J., Hanfland, R.A., Smith, T.L., Samuel, M., Wax, D., Murphy, C.N., Rieke, A., Whitworth, K., Uc, A., Starner, T.D., Brogden, K.A., Shilyansky, J., McCray, P.B., Zabner, J., Prather, R.S., Welsh, M.J. :
Disruption of the CFTR gene produces a model of cystic fibrosis in newborn pigs. Science 321:1837-41, 2008. Pubmed reference: 18818360. DOI: 10.1126/science.1163600.
2006 Maurisse, R., Cheung, J., Widdicombe, J., Gruenert, D.C. :
Modification of the pig CFTR gene mediated by small fragment homologous replacement. Ann N Y Acad Sci 1082:120-3, 2006. Pubmed reference: 17145933. DOI: 10.1196/annals.1348.063.
1991 Gasparini, P., Nunes, V., Dognini, M., Estivill, X. :
High conservation of sequences involved in cystic fibrosis mutations in five mammalian species. Genomics 10:1070-2, 1991. Pubmed reference: 1717363.